What is cystic fibrosis?

Cystic Fibrosis

Cystic fibrosis in children: Overview

Cystic fibrosis is a lifelong illness that causes mucus to become thick and sticky. Mucus is the slippery substance inside the nose, throat, sinuses, and airways. With this condition, the mucus builds up and clogs passages in the body. The mucus can lead to serious breathing problems and lung disease. When it affects the pancreas, it can lead to malnutrition, diabetes, and problems with a child's growth and development. It also can lead to severe constipation and bowel blockage.

Treatments can include lung therapy, medicines, exercise, and diet changes. Some people who have this condition need to take enzymes and vitamins. There isn't a cure for cystic fibrosis. But early diagnosis and new treatments are helping people live longer.

You may be worried about how to care for your child. It is normal to feel overwhelmed. But your child's care team will help you learn to do all that your child needs.

Cystic fibrosis (CF)

Cystic fibrosis (CF) is a chronic and progressive disease, often diagnosed in childhood, that causes mucus to become thick and sticky. The mucus builds up and clogs passages in the lungs, pancreas, and many other organs in the body.

There is no cure for CF, but early diagnosis and new treatments are helping people live longer. Management of the disease varies from person to person and generally focuses on treating respiratory and digestive problems to prevent infection and other problems.

What happens when you have cystic fibrosis (CF)?

CF causes mucus in the body to become thick and sticky. The mucus buildup can cause problems in many of the body's organs and systems. These include:

Lungs and respiratory system.

People with CF have thick and sticky mucus that traps bacteria. This causes lots of lung infections and often causes permanent lung damage. Bronchiectasis, which is caused by long-lasting airway inflammation, is common.

Pancreas and digestive system.

Mucus can interfere with how the pancreas works. This can make it hard for the child to absorb nutrients from food.

Sweat glands.

You may notice that your child has unusually salty skin. CF can cause a person to become easily dehydrated or to have very low salt levels.

Reproductive organs.

Almost all men who have cystic fibrosis are unable to father a child. Women who have CF may have more difficulty getting pregnant than other women.

Skeletal system.

People who have CF may have weaker bones than other people. This is because their bones contain lower levels of minerals. Weakened bones can lead to bone fractures or osteoporosis. CF can also cause swollen or painful joints (arthropathy or arthritis). These problems are more common in adults than in children.

Life expectancy

The life expectancy for people with CF has been steadily increasing. Because of newborn screening, many babies with CF are diagnosed before they develop symptoms. Early diagnosis and personalized treatment can help them feel better and live longer.

What are the symptoms of cystic fibrosis (CF)?

Some common symptoms in a baby who has CF include a blocked small intestine, breathing problems, not growing or gaining weight even with a good appetite, salty skin, and diarrhea. Other symptoms in children may include clubbing (rounding and flattening) of the fingers and growths in the nose or sinuses.

How is cystic fibrosis (CF) treated?

Treatment depends on what kinds of health problems CF is causing. It also depends on how your child's body responds to different types of treatment. Doctors often recommend that people who have CF follow a personal treatment plan that includes airway clearance techniques, medicines, a fitness plan, and nutritional therapy.

What are the risks of a lung transplant for cystic fibrosis?

The main risks of lung transplant are problems during the surgery and rejection of the donated organs. Infection and rejection of transplanted organs have similar symptoms, including fever, tiredness, and trouble breathing.

After a lung transplant, preventive antibiotic therapy starts right away. You will also take immunosuppressant medicines, usually for the rest of your life. Although immunosuppressant medicines raise the chances that your body will accept the new lungs, they also make it harder for your body to fight off infection.

How is cystic fibrosis (CF) diagnosed?

A medical history and a physical exam are often the first steps in diagnosing CF. This is followed by screening or lab tests.

The diagnosis of CF requires one of the following:

  • Early symptoms of CF
  • A brother or sister who has CF
  • A positive newborn screening test

Also, there must be at least one of the following:

  • A genetic test showing that a person inherited a changed gene (known as the CFTR gene) from each parent.
  • A positive sweat test showing a higher-than-normal level of chloride in sweat.
  • An abnormal nasal potential difference test. This test uses electrodes on the lining of the nose to see how well salts flow into and out of cells.

How are medicines used to treat cystic fibrosis (CF)?

Medicines for CF help keep the lungs as healthy as possible.

You may need to take medicines to:

  • Treat infections.
  • Open airways in the lungs or keep them open.
  • Control the amount and thickness of mucus.
  • Reduce inflammation.
  • Replace the effect of digestive enzymes.
  • Improve lung function.

Some medicines work better for some people than for others. A medicine that works well for one person may not work for someone else. It can take time to find the medicines that work best for you or your child.

How can you care for your child who has cystic fibrosis (CF)?

  • Give your child foods that meet your child's special nutritional needs. A dietitian can help you plan meals.
  • Encourage your child to exercise. It can make your child feel better. Talk to your child's doctor about how your child can be active.
  • Encourage your child to drink lots of fluids. People who have CF need extra fluids. Ask your doctor how much fluid your child needs.
  • Follow your doctor's directions to keep your child's airway clear.
  • Make sure your child gets all the recommended childhood vaccines, as well as the pneumococcal vaccine.
  • Keep your child away from smoke. Do not smoke or let anyone else smoke around your child or in your house.

Handling the challenges of CF

You and your child will struggle at times with this illness. But having a good attitude and lots of support will help you and your child cope.

  • Focus on your child's strengths. Let your child know that you love and believe in them.
  • Give your child some responsibility for their own care. Children who have a say in their treatment often stay healthier.
  • Learn about the disease. This will help you figure out what you can do to help your child.
  • Do the best you can and know that you cannot control everything.

How is surgery used to treat cystic fibrosis (CF)?

Surgery may be needed to treat problems from CF. These procedures may include:

  • Lung transplant. It may be done for people who have severe lung disease. Not everyone is a good candidate for a lung transplant.
  • Chest tube drainage and possible thoracoscopy. This is done to treat a collapsed lung (pneumothorax).
  • Repair of an intestine that has collapsed inside itself or removal of a bowel obstruction.
  • Removal of nasal polyps or endoscopic sinus surgery.

If a child has CF and isn't able to get all the nutrients they need from food, a feeding tube may be placed in the child's stomach.

Cystic Fibrosis: What It Means for You and Your Child

What increases the risk for cystic fibrosis (CF)?

CF is a genetic condition. It is an autosomal recessive disease. This means that to get the disease, you must inherit the changed (mutated) gene from both parents. Keep in mind:

  • If a person inherits the changed gene from only one parent, they will not get CF but will be a carrier of the disease. Carriers may pass the changed gene on to their children.
  • If you are planning to get pregnant, talk to your doctor about having a genetic test to find out your chances of having a child with CF.

Organs Most Frequently Affected by Cystic Fibrosis

Organs most frequently affected by cystic fibrosis (lungs and pancreas)

Cystic fibrosis causes mucus to become thick and sticky, which can block the normal function of the organs. Most often, the lungs and pancreas are affected.

What is cystic fibrosis (CF)?

Cystic fibrosis (CF) is a common genetic disease that causes mucus in the body to become thick and sticky. The mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas. People who have CF can have serious breathing problems and lung disease.

What causes cystic fibrosis (CF)?

CF is caused by a change, or mutation, in a gene. The changed gene is passed down in families. To pass on this disease, both parents must be carriers of the changed gene.

How can you help clear mucus from cystic fibrosis (CF) from your child's lungs?

CF causes mucus to become thick and sticky, which can clog the lungs and cause serious problems. You can help your child maintain lung function and avoid problems from mucus buildup and blockage by doing airway clearance techniques. As your child gets older, they'll be able to do this on their own.

Airway clearance techniques (ACT) include special breathing and coughing exercises. It may involve using postural drainage and percussion. An older child or adult may use a percussive vest or use a positive expiratory pressure (PEP) device. These all help with clearing mucus from the lungs.

Your child's care team will make a personal treatment plan that includes ACT. These therapies are one part of a larger treatment plan to help keep your child's lungs clear.

It is important for your child to visit their doctor regularly and make any needed changes in treatment.

Cystic fibrosis: When to call

Call 911 anytime you think you may need emergency care. For example, call if:

  • You have severe trouble breathing.

Call your doctor now or seek immediate medical care if:

  • You have wheezing or coughing that is new or gets worse.
  • You have a fever.
  • You lose weight or lose your appetite.
  • You have severe belly pain or vomiting.
  • You cough up or vomit blood.
  • You start to urinate more often, are often thirsty, or have blurred vision.

Watch closely for changes in your health, and be sure to contact your doctor if:

  • You have more trouble breathing than usual.
  • You have constipation that is new or gets worse.

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